Suenaga Shinta, Ichiyanagi Osamu, Ito Hiromi, Naito Sei, Kato Tomoyuki, Nagaoka Akira, Kato Tomoya, Yamakawa Mitsunori, Obara Yutaro, Tsuchiya Norihiko
Department of Urology, Yamagata University Faculty of Medicine, Japan.
Intern Med. 2016;55(24):3611-3621. doi: 10.2169/internalmedicine.55.7293. Epub 2016 Dec 15.
Composite pheochromocytoma (cPC) is extremely rare, arising in the adrenal medulla as a mixture of PC and other tumors of neural origin. We herein report on a case of adrenal incidentaloma post-operatively diagnosed as cPC with ganglioneuroblastoma (GNBL). The PC component had 7 points on the PASS, a Ki-67 index of 5.1%, a focal absence of sustentacular cells, and no genetic aberrations in succinate dehydrogenase subunit B. The GNBL component exhibited no N-myc amplification. Tumor cells of both components were stained positively for extracellular signal-regulated kinase 5 and ankyrin repeat domain 1. The aberrant activation of growth signaling may play a role in the marginal malignancy of cPC.
复合性嗜铬细胞瘤（cPC）极为罕见，起源于肾上腺髓质，是嗜铬细胞瘤（PC）与其他神经源性肿瘤的混合体。我们在此报告一例肾上腺意外瘤，术后诊断为合并神经母细胞瘤（GNBL）的cPC。PC成分在PASS上有7分，Ki-67指数为5.1%，局灶性支持细胞缺失，琥珀酸脱氢酶亚基B无基因畸变。GNBL成分未表现出N-myc扩增。两种成分的肿瘤细胞细胞外信号调节激酶5和锚蛋白重复结构域1染色均为阳性。生长信号的异常激活可能在cPC的边缘性恶性中起作用。
